{
  "abstract": "Introduction Cryofibrinogenemia (CF) is a rare disorder in which plasma protein complexes precipitate at low temperatures and redissolve upon warming. Although described in association with various autoimmune and vascular diseases, its clinical significance remains unclear. In systemic sclerosis (SSc), systematic studies investigating the prevalence of CF and its potential correlations with disease manifestations are scarce.Material and Methods We aimed to assess the frequency of CF in a cohort of SSc patients and explore associations with clinical, serological, and therapeutic features, independently of disease subtype.We retrospectively analyzed SSc patients followed at our tertiary center, diagnosed according to the 2013 ACR/EULAR criteria, and tested for CF between February 2020 and July 2025. Demographic, clinical, immunological, and therapeutic data were collected. Categorical variables were compared using chi-square or Fisher’s exact test. Estimated systolic pulmonary arterial pressure (sPAP) was assessed by echocardiography.Results A total of 184 patients were included, predominantly female (88%) and with limited cutaneous SSc (78.8%). CF was detected in 52.7% of patients, whereas coexistence with cryoglobulins occurred in 9.2%. No significant differences were observed between patients with and without CF regarding age at diagnosis, sex distribution, peripheral vascular manifestations, or ongoing therapies, suggesting that CF occurrence is independent of these demographic, clinical, and treatment-related factors. Interestingly, no increased prevalence of malignancy was observed among patients with CF, which differs from some reports in the literature. CF positivity was significantly associated with rheumatoid factor positivity (p = 0.038), interstitial lung disease (p = 0.030), and sPAP > 30 mmHg (p = 0.003) and > 35 mmHg (p = 0.031). A trend toward higher all-cause mortality was observed (p = 0.07). When cryocrit was > 1%, associations with sPAP > 30 mmHg (p = 0.001) and mortality (p = 0.04) were stronger.Conclusions Isolated CF is a frequent finding in SSc, considerably more common than cryoglobulinemia, and this is the first study where it associated with interstitial lung disease and pulmonary arterial hypertension. These findings suggest that CF, often clinically silent, may serve as a marker of vascular and pulmonary involvement. Ongoing analyses aim to expand the patient cohort and incorporate additional clinical and serological variables to further characterize the relationship between CF and pulmonary vascular or interstitial involvement.Abstract P.383 Table 1",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Policlinico of Modena, Modena, Italy"
      ],
      "name": "Luca Keller Sarmiento"
    },
    {
      "affiliations": [
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy"
      ],
      "name": "Caterina Vacchi"
    },
    {
      "affiliations": [
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy"
      ],
      "name": "Gabriele Amati"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Policlinico of Modena, Modena, Italy",
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy"
      ],
      "name": "Benedetta Bongiovanni"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Policlinico of Modena, Modena, Italy",
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy"
      ],
      "name": "Amelia Spinella"
    },
    {
      "affiliations": [
        "Department of Laboratory Medicine and Pathological Anatomy, Azienda Ospedaliero- Universitaria E Azienda USL Di Modena,, Baggiovara, Italy"
      ],
      "name": "Patrizia Natali"
    },
    {
      "affiliations": [
        "Department of Laboratory Medicine and Pathological Anatomy, Azienda Ospedaliero- Universitaria E Azienda USL Di Modena,, Baggiovara, Italy"
      ],
      "name": "Daria Debbia"
    },
    {
      "affiliations": [
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy"
      ],
      "name": "Martina Orlandi"
    },
    {
      "affiliations": [
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy"
      ],
      "name": "Ottavio Secchi"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Policlinico of Modena, Modena, Italy",
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy"
      ],
      "name": "Marco De Pinto"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Scleroderma Unit, University Hospital of Modena, Policlinico of Modena, Modena, Italy",
        "Rheumatology Unit, University of Modena and Reggio Emilia, Medical School, Modena, Italy",
        "Department of Maternal, Child and Adult Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena, Italy"
      ],
      "name": "Dilia Giuggioli"
    }
  ],
  "title": "P.383 Unveiling cryofibrinogenemia in systemic sclerosis: novel links to lung disease and pulmonary hypertension",
  "uid": "2d134c97-7ea0-5d0f-a8a5-521a340cc0b5"
}